Mycosis fungoides (MF) and Sézary's syndrome (SS) are cutaneous T-cell lymphomas characterized ultrastructurally by the presence of lymphoid cells with deep and narrow nuclear indentations (cerebriform mononuclear cells or CMC). Early diagnosis of MF and SS is difficult because in their early stages they often resemble various forms of chronic, benign skin lesions. By measuring the frequency distribution of a nuclear shape parameter (nuclear contour index or NCI) of lymphoid cells in skin infiltrates using computer assisted planimetry, we tried to classify suspect cases into the malignant and benign groups. From 12 patients with MF or SS (malignant group) and 11 patients with chronic, benign skin lesions (benign group) the frequency distribution of the NCI of the lymphoid cell population was measured. Nonlinear discriminant analysis selected the 70th and 25th percentile of the NCI distribution of the lymphoid cells in the skin infiltrates as parameters by which these patients could be classified correctly into the malignant or benign groups with a probability of over 95%. The predictive value of these parameters was tested on ten patients suspected of having cutaneous T-cell lymphomas. Three cases were classified as benign and 7 as malignant. In a three-year follow up cutaneous T-cell lymphomas did not develop in any of the 3 cases classified as benign, MF developed in 5 of 7 cases classified as malignant, 1 patient has lymphomatoid papulosis and 1 patient is still suspect for MF. These results are compared with those of DNA cytophotometry performed on skin imprint preparations. It is concluded that morphometry of lymphoid cells in skin lesions of patients suspect for MF and SS can make an important contribution to an early diagnosis of MF or SS.

Two boys are described with congenital microcephaly, infantile spasms, psychomotor retardation and an early-onset nephrotic syndrome. The autopsy findings of one patient are described in detail. Polymicrogyria was the most prominent feature and the kidneys showed focal segmental glomerulosclerosis. These findings have been described as a clinical entity, the leading symptoms being congenital microcephaly, early-onset nephrotic syndrome and mental retardation, accompanied by various other clinical symptoms. A review of the literature suggests an autosomal recessive mode of inheritance.

For 26 patients with mycosis fungoides (MF), the type and extent of the skin lesions, the percentage of cerebriform mononuclear cells (CMC), and T and B lymphocytes in the peripheral blood and lymph nodes were correlated with MF involvement of regional lymph nodes, the clinical course, and response to therapy. Skin tumors and an involvement of more than 25% of the skin correlated well with lymph node involvement. Normal percentages (2--18%) of CMC in the peripheral blood were found for MF patients without lymph node involvement when compared with those found for patients with benign erythroderma and healthy donors. Elevated circulating CMC percentages (greater than 20%) were observed in 9 of 11 MF patients with lymph node involvement. In the lymph node cell suspensions from nine of ten MF patients with lymph node involvement, increased CMC values were found (greater than 15%), whereas two of three MF patients without lymph node involvement showed percentages comparable (4% and 7%, respectively) with those of the control lymph nodes. In the peripheral blood of patients with MF, decreased percentages of T cells (less than or equal to 55%) were found predominantly for patients with lymph node involvement, whereas normal percentages were noted for most of the patients without lymph node involvement. No consistent differences in the percentage of T and B cells in the lymph node cell suspensions were found between MF patients with and without lymph node involvement. Patients with lymph node involvement showed in general a partial response to therapy with an unfavorable clinical course in contrast to patients without lymph node involvement. Increased percentages of CMC (greater than 20%) and decreased percentages of T cells (less than or equal to 55%) in the peripheral blood, the presence of skin tumors, and involvement of more than 25% of the skin are prognostically unfavorable signs for patients with mycosis fungoides.

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